Mitterhofer A P, Bonomo L
Istituto di Clinica Medica III, Università La Sapienza, Roma.
Recenti Prog Med. 1994 May;85(5):289-95.
The adult Still's disease (ASD) is an uncommon inflammatory systemic disorder which affects the young adult. It is characterized by high spiking fever, vanishing rash, oligopolyarthritis, neutrophilic leucocytosis, negative titers for rheumatoid factor and antinuclear antibodies. Polyserositis, sore throat, uveitis are sometimes present and in one third of the cases it is possible to find hepato-splenomegaly with lymph node enlargement. G. Still first described the disease in child, in 1897, and in the adult it was recognized as a nosologic entity more than 70 years later. The ASD diagnosis is difficult and it is possible after the exclusion of many other diseases. Clinical manifestation are all nonspecific. In particular the presence of adenopathy, hepato-splenomegaly may suggest the possibility of a malignant lymphoma. Important exclusions include many other diseases such as the rheumatic fever, periodic fever, Lyme disease. At the same time a probable diagnosis of ASD should be considered in all the cases of high fever with rash, arthritis, neutrophilic leucocytosis or in the cases of fever of unknown origin (FUO). The prognosis is considered overall benign. The disease is usually sensible to salicylate treatment, even but the association with corticosteroids or, sometimes, with cytotoxic therapy is often required.
成人斯蒂尔病(ASD)是一种影响青年成人的罕见炎症性全身性疾病。其特征为高热、皮疹消退、少关节炎、中性粒细胞增多、类风湿因子和抗核抗体滴度阴性。有时会出现多浆膜炎、咽痛、葡萄膜炎,三分之一的病例可发现肝脾肿大伴淋巴结肿大。G. 斯蒂尔于1897年首次描述了儿童期的这种疾病,70多年后成人期的该疾病才被确认为一个病种。ASD的诊断困难,需排除许多其他疾病后才有可能确诊。临床表现均无特异性。特别是存在腺病、肝脾肿大可能提示恶性淋巴瘤的可能性。重要的排除疾病包括许多其他疾病,如风湿热、周期性发热、莱姆病。同时,对于所有伴有皮疹、关节炎、中性粒细胞增多的高热病例或不明原因发热(FUO)病例,都应考虑ASD的可能诊断。总体预后被认为是良性的。该疾病通常对水杨酸盐治疗敏感,但常需要联合使用皮质类固醇,有时还需要联合细胞毒性疗法。